Long-term prognosis of infantile spasms after standard ACTH treatment in Japan.

概要

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In patients with infantile spasms, ACTH therapy was carried out according to Fukuyama's method which is the generally accepted method in Japan. The long term prognosis in these patients was then examined. The patients whose course could be observed for 5-24 years (11 years on the average) after the therapy were divided into the cryptogenic (C) group and the symptomatic (S) group. On the basis of the initial therapy, they were further divided into C1, S1, C2 and S2. In the former two groups ACTH-Z was consecutively administered at a daily dose of 0.25mg (10 units) to 0.5 mg (20 units) for more than 14 days and in the latter two groups the drug was given in the same way as the former groups at smaller daily doses for less than 14 days, or other anticonvulsant drugs were given. C1, C2, S1 and S2 consisted of 13, 9, 4 and 12 patients, respectively. At present 8% of C1, 11% of C2, 50% of S1 and 58% of S2 have seizures, and 69% of C1, 44% of C2, none of S1 and 8% of S2 are in or have graduated from normal school. The prognosis of seizures and educational abilities of C1 patients were favorable and approximately the same as those following longterm therapy by massive administration of ACTH in western countries. The difference in prognosis between the patients in the Ci group and those following lower doses and shorter duration of ACTH therapy (a part of C2 patients) should be studied further.
一般社団法人日本小児神経学会の論文